165 results match your criteria Sickle Cell Anemia Skeletal


Sickle cell diseases: What can nuclear medicine offer?

Hell J Nucl Med 2019 Jan-Apr;22(1):2-3. Epub 2019 Mar 7.

Nuclear Medicine Unit, Interdisciplinary Department of Medicine, University of Bari "Aldo Moro", Bari, Italy.

Sickle cell disease (SCD) is the best known haemoglobinopathy, caused by a mutation substituting valina for glutamic acid at position 6 of the beta-globin chain of adult hemoglobin A, resulting in hemoglobin S (HbS). The homozygous HbS disease (HbSS), an autosomal recessive disorder, is the most common form and the Mediterranean area, along with sub-Saharian African and India, have the highest prevalence (1%-15%). In particular, Sicily with a prevalence of 2%-5%, is among the most interested regions. Read More

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http://dx.doi.org/10.1967/s002449910950DOI Listing
March 2019
1 Read

A microfluidic approach to study the effect of mechanical stress on erythrocytes in sickle cell disease.

Lab Chip 2018 09;18(19):2975-2984

Biologie Intégrée du Globule Rouge UMR_S1134, Inserm, Univ. Paris Diderot, Sorbonne Paris Cité, Univ. de la Réunion, Univ. des Antilles, INTS, 6 rue Alexandre Cabanel, 75015 Paris, France.

The human red blood cell is a biconcave disc of 6-8 × 2 μm that is highly elastic. This capacity to deform enables it to stretch while circulating through narrow capillaries to ensure its main function of gas exchange. Red cell shape and deformability are altered in membrane disorders because of defects in skeletal or membrane proteins affecting protein-protein interactions. Read More

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http://xlink.rsc.org/?DOI=C8LC00637G
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http://dx.doi.org/10.1039/c8lc00637gDOI Listing
September 2018
31 Reads

The role of nitrite in muscle function, susceptibility to contraction injury, and fatigability in sickle cell mice.

Nitric Oxide 2018 11 14;80:70-81. Epub 2018 Aug 14.

Department of Perioperative Medicine, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD, 20892, USA. Electronic address:

Sickle cell disease (SCD) patients can have limited exercise capacity and muscle dysfunction characterized by decreased force, atrophy, microvascular abnormalities, fiber distribution changes, and skeletal muscle energetics abnormalities. Growing evidence suggests that in SCD there is alteration in nitric oxide (NO) availability/signaling and that nitrate/nitrite can serve as a NO reservoir and enhance muscle performance. Here, we examined effects of nitrite on muscle strength, exercise capacity, and on contractile properties of fast-(extensor digitorum longus, EDL) and slow-twitch (soleus) muscles in SCD mice. Read More

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http://dx.doi.org/10.1016/j.niox.2018.08.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186197PMC
November 2018
1 Read
3.520 Impact Factor

How Sickle Cell Disease Impairs Skeletal Muscle Function: Implications in Daily Life.

Med Sci Sports Exerc 2019 Jan;51(1):4-11

Inter-university Laboratory of Human Movement Sciences, University of Savoie Mont Blanc, Chambéry, FRANCE.

Sickle cell disease (SCD) is the most frequent life-threatening genetic hemoglobinopathy in the world and occurs due to the synthesis of abnormal hemoglobin S (HbS). hemoglobin S-containing red blood cells (RBC) are fragile, leading to hemolysis and anemia, and adhere to the endothelium, leading to hemorheological and hemodynamical disturbances. In its deoxygenated form, HbS may polymerize, leading to sickling of red blood cells and potentially to vasoocclusive crises. Read More

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http://dx.doi.org/10.1249/MSS.0000000000001757DOI Listing
January 2019
4 Reads

Muscle dysfunction is associated with poorer health-related quality of life in adults with sickle cell anaemia.

J Back Musculoskelet Rehabil 2019 ;32(1):43-53

Rehabilitation Sciences Post-Graduation Programme, Augusto Motta University, Rio de Janeiro, Brazil.

Background: It is known that episodes of microvascular obstruction and oxidative stress in sickle cell anaemia (SCA) can damage muscle tissue. As a consequence, deterioration in muscle function may potentially contribute to poor health-related quality of life (HRQoL) in subjects with SCA, particularly those who do not use long-term treatment.

Objectives: To evaluate muscle function in adults with SCA, to study the correlations between muscle function and HRQoL and to analyse the impact of hydroxyurea treatment. Read More

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http://dx.doi.org/10.3233/BMR-171027DOI Listing

Bone and blood interactions in human health and disease.

Bone 2019 Feb 21;119:65-70. Epub 2018 Feb 21.

Division of Endocrinology, Stanford University School of Medicine, Stanford, CA, United States. Electronic address:

Under physiologic conditions hematopoiesis takes place in the bone marrow, and the skeleton provides the structural and supportive network necessary for normal hematopoiesis. Chronic disorders affecting hematopoiesis such as sickle cell anemia and thalassemia demonstrate striking skeletal phenotypes including bone loss and increased fracture risk. There is mounting evidence that anemia in older populations may also be associated with bone fragility. Read More

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http://dx.doi.org/10.1016/j.bone.2018.02.019DOI Listing
February 2019
2 Reads

Differential diagnostic perspectives provided by en face microscopic examination of articular surface defects.

Clin Rheumatol 2018 Mar 4;37(3):831-836. Epub 2018 Feb 4.

Department of Vertebrate Paleontology, Carnegie Museum, Pittsburgh, PA, 15213, USA.

Surface defects have a central position in diagnosis of articular pathology. Recognizing the limitations of standard radiologic techniques and those imposed by positioning and averaging artifacts on CT evaluation, direct visualization of surface defects was pursued to identify disease characteristics that would facilitate interpretation of radiologic findings. Epi-illumination surface microscopy was utilized to examine macroscopically recognized articular surface defects in individuals in the Hamann-Todd, Terry, and Huntington human skeletal collections with previously verified diagnoses of rheumatoid arthritis, spondyloarthropathy, juvenile inflammatory arthritis (JIA), calcium pyrophosphate deposition disease (CPPD), gout, metastatic cancer, multiple myeloma, septic arthritis, tuberculosis, fungal arthritis, histiocytosis and sickle cell anemia (Rothschild and Rothschild Clin Infect Dis 20(5):1402-1408, 1995; Rothschild et al. Read More

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http://dx.doi.org/10.1007/s10067-018-4001-xDOI Listing
March 2018
16 Reads

Endurance training reduces exercise-induced acidosis and improves muscle function in a mouse model of sickle cell disease.

Mol Genet Metab 2018 03 28;123(3):400-410. Epub 2017 Nov 28.

Aix-Marseille Univ, CNRS, CRMBM, Marseille, France.

Sickle cell disease (SCD) mice (Townes model of SCD) presented exacerbated exercise-induced acidosis and fatigability as compared to control animals. We hypothesize that endurance training could represent a valuable approach to reverse these muscle defects. Endurance-trained HbAA (HbAA-END, n=10), HbAS (HbAS-END, n=11) and HbSS (HbSS-END, n=8) mice were compared to their sedentary counterparts (10 HbAA-SED, 10 HbAS-SED and 9 HbSS-SED mice) during two rest - exercise - recovery protocols during which muscle energetics and function were measured. Read More

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http://dx.doi.org/10.1016/j.ymgme.2017.11.010DOI Listing
March 2018
11 Reads

Skeletal and myocardial microvascular blood flow in hydroxycarbamide-treated patients with sickle cell disease.

Br J Haematol 2017 11 7;179(4):648-656. Epub 2017 Sep 7.

Knight Cardiovascular Institute, Oregon Health and Science University, Portland, OR, USA.

In sickle cell disease (SCD), abnormal microvascular function combined with chronic anaemia predisposes patients to perfusion-demand mismatch. We hypothesized that skeletal muscle and myocardial perfusion, normalized to the degree of anaemia, is reduced at basal-state compared to controls, and that this defect is ameliorated by hydroxycarbamide (HC; also termed hydroxyurea) therapy. Twenty-one SCD patients, of whom 15 were treated with HC, and 27 controls underwent contrast-enhanced ultrasound (CEU) perfusion imaging of the forearm as well as the myocardium. Read More

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http://dx.doi.org/10.1111/bjh.14918DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5696022PMC
November 2017
32 Reads

Exacerbated in vivo metabolic changes suggestive of a spontaneous muscular vaso-occlusive crisis in exercising muscle of a sickle cell mouse.

Blood Cells Mol Dis 2017 06 11;65:56-59. Epub 2017 May 11.

Aix-Marseille Univ, CNRS, CRMBM, Marseille, France.

While sickle cell disease (SCD) is characterized by frequent vaso-occlusive crisis (VOC), no direct observation of such an event in skeletal muscle has been performed in vivo. The present study reported exacerbated in vivo metabolic changes suggestive of a spontaneous muscular VOC in exercising muscle of a sickle cell mouse. Using magnetic resonance spectroscopy of phosphorus 31, phosphocreatine and inorganic phosphate concentrations and intramuscular pH were measured throughout two standardized protocols of rest - exercise - recovery at two different intensities in ten SCD mice. Read More

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http://dx.doi.org/10.1016/j.bcmd.2017.05.006DOI Listing
June 2017
4 Reads

Moderate and intense muscular exercises induce marked intramyocellular metabolic acidosis in sickle cell disease mice.

J Appl Physiol (1985) 2017 May 9;122(5):1362-1369. Epub 2017 Mar 9.

Aix-Marseille Univ, CNRS, CRMBM, Marseille, France; and.

Sickle cell disease (SCD) is associated with an impaired oxygen delivery to skeletal muscle that could alter ATP production processes. The present study aimed to determine the effects of sickle hemoglobin (HbS) on muscle pH homeostasis in response to exercise in homozygous (HbSS, = 9) and heterozygous (HbAS, = 10) SCD (Townes) mice in comparison to control (HbAA, = 10) littermates. Magnetic resonance spectroscopy of phosphorus 31 enabled to measure intramuscular pH and phosphocreatine (PCr) concentration during rest-stimulation-recovery protocols at two different intensities. Read More

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http://dx.doi.org/10.1152/japplphysiol.01099.2016DOI Listing
May 2017
15 Reads

Myonecrosis in Sickle Cell Anemia: Case Study.

Am J Case Rep 2017 Jan 30;18:100-103. Epub 2017 Jan 30.

Department of Podiatry, Advocate Illinois Masonic Hospital/Stroger Hospital of Cook County, Chicago, IL, USA.

BACKGROUND Myonecrosis is one of the more poorly studied, painful manifestations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of such symptoms. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and myonecrosis. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5292993PMC
January 2017
8 Reads

Impaired muscle force production and higher fatigability in a mouse model of sickle cell disease.

Blood Cells Mol Dis 2017 03 11;63:37-44. Epub 2017 Jan 11.

Aix-Marseille Univ, CNRS, CRMBM, Marseille, France.

Skeletal muscle function has been scarcely investigated in sickle cell disease (SCD) so that the corresponding impact of sickle hemoglobin is still a matter of debate. The purpose of this study was to investigate muscle force production and fatigability in SCD and to identify whether exercise intensity could have a modulatory effect. Ten homozygous sickle cell (HbSS), ten control (HbAA) and ten heterozygous (HbAS) mice were submitted to two stimulation protocols (moderate and intense) to assess force production and fatigability. Read More

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http://dx.doi.org/10.1016/j.bcmd.2017.01.004DOI Listing
March 2017
4 Reads

Muscle IL1β Drives Ischemic Myalgia via ASIC3-Mediated Sensory Neuron Sensitization.

J Neurosci 2016 06;36(26):6857-71

Department of Anesthesia, Division of Pain Management, Cincinnati Children's Hospital Medical Center, and Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio 45229

Unlabelled: Musculoskeletal pain is a significantly common clinical complaint. Although it is known that muscles are quite sensitive to alterations in blood flow/oxygenation and a number of muscle pain disorders are based in problems of peripheral perfusion, the mechanisms by which ischemic-like conditions generate myalgia remain unclear. We found, using a multidisciplinary experimental approach, that ischemia and reperfusion injury (I/R) in male Swiss Webster mice altered ongoing and evoked pain-related behaviors in addition to activity levels through enhanced muscle interleukin-1 beta (IL1β)/IL1 receptor signaling to group III/IV muscle afferents. Read More

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http://dx.doi.org/10.1523/JNEUROSCI.4582-15.2016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4926236PMC
June 2016
16 Reads

Young Man With Bilateral Arm Pain.

Ann Emerg Med 2016 Jun;67(6):e21-e22

Department of Emergency Medicine, McGill University Health Centre, Montreal, Quebec, Canada.

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http://dx.doi.org/10.1016/j.annemergmed.2015.10.023DOI Listing
June 2016
1 Read

Comment on: negative health implications of sickle cell trait in high income countries: from the football field to the laboratory.

Authors:
Samir K Ballas

Br J Haematol 2016 10 2;175(2):349-350. Epub 2015 Dec 2.

Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA.

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http://dx.doi.org/10.1111/bjh.13860DOI Listing
October 2016
40 Reads

Is MRI Necessary for Skeletal Evaluation in Sickle Cell Disease.

J Clin Diagn Res 2015 Jun 1;9(6):TC08-12. Epub 2015 Jun 1.

Senior Resident, Department of Internal Medicine, AIIMS , Rishikesh, Uttarakhand, India .

Background: More than 50% of the world's cases of sickle cell anaemia are in India with an estimated population of 1.27 billion as against estimated world's population of 7.24 billion. Read More

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http://dx.doi.org/10.7860/JCDR/2015/12747.6095DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4525574PMC
June 2015
3 Reads

Short term results of cementless total hip arthroplasty in sicklers.

Indian J Orthop 2015 Jul-Aug;49(4):447-51

Department of Orthopaedics, Joint Reconstruction, Sports Injury and Spine, BLK Super Speciality Hospital, New Delhi, India.

Background: Sickle cell (SC) disease leading to endarteritis induces skeletal changes in the form of osteitis, sclerosis of femoral canal and osteonecrosis of the femoral head. All these make total hip arthroplasty (THA) difficult and prolonged. There is increased risk of infection, SC crisis and increased complication rate. Read More

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http://dx.doi.org/10.4103/0019-5413.159659DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510800PMC
August 2015
2 Reads

Abnormal Regulation of Microvascular Tone in a Murine Model of Sickle Cell Disease Assessed by Contrast Ultrasound.

J Am Soc Echocardiogr 2015 Sep 27;28(9):1122-8. Epub 2015 Jun 27.

Knight Cardiovascular Institute, Oregon Health & Science University, Portland, Oregon. Electronic address:

Background: Microvascular dysregulation, abnormal rheology, and vaso-occlusive events play a role in the pathophysiology of sickle cell disease (SCD). The aim of this study was to test the hypothesis that abnormalities in skeletal muscle perfusion in a murine model of SCD could be parametrically assessed by quantitative contrast-enhanced ultrasound perfusion imaging.

Methods: A murine model of moderate SCD without anemia produced by homozygous β-globin deletion replaced by human βs-globin transgene (NY1DD-/-; n = 18), heterozygous transgene replacement (NY1DD+/-; n = 19), and C57Bl/6 control mice (n = 14) was studied. Read More

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http://dx.doi.org/10.1016/j.echo.2015.05.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4567487PMC
September 2015
20 Reads

Bioelectric impedance analysis of body composition of children and adolescents with sickle cell anemia in Enugu, Nigeria.

Pediatr Hematol Oncol 2015 May 14;32(4):258-68. Epub 2015 Apr 14.

Department of Paediatrics, College of Medicine, University of Nigeria Teaching Hospital, University of Nigeria , Ituku-Ozalla, Enugu , Nigeria.

Background: Body composition indices are widely used to evaluate growth and nutrition in children, particularly those with sickle cell anemia (SCA), who are known to have impaired growth, impaired skeletal maturation, and delayed puberty. The current study is aimed at determining the body composition of children with SCA.

Methods: Consecutive selection of SCA children aged 6 to 18 years, who served as subjects and their age and gender matched children with HbAA (controls), selected using multi-stage systematic sampling. Read More

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http://dx.doi.org/10.3109/08880018.2015.1010111DOI Listing
May 2015
10 Reads

Evidence for a profound remodeling of skeletal muscle and its microvasculature in sickle cell anemia.

Am J Pathol 2015 May 13;185(5):1448-56. Epub 2015 Mar 13.

Laboratory of Exercise Physiology EA4338, Savoie Mont Blanc University, Chambéry, France. Electronic address:

Sickle cell anemia (SCA) is a hemoglobinopathy leading to major hematologic, hemorheologic, and hemodynamic disorders that induce various complications, including organ failure, and ultimately lead to death. Here, we assessed for the first time repercussions of SCA on skeletal muscle and its microvasculature. Twenty-seven sedentary Cameroonian volunteer men participated in the study. Read More

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http://dx.doi.org/10.1016/j.ajpath.2015.01.023DOI Listing
May 2015
4 Reads

[Muscle involvement in sickle cell disease].

Med Clin (Barc) 2015 Nov 7;145(9):413-4. Epub 2015 Feb 7.

Unidad de Enfermedades Neuromusculares, Servicio de Neurología, Hospital del Mar, Barcelona, España.

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http://dx.doi.org/10.1016/j.medcli.2014.12.010DOI Listing
November 2015
3 Reads

Vitamin D deficiency and acute vaso-occlusive complications in children with sickle cell disease.

Pediatr Blood Cancer 2015 Apr 13;62(4):643-7. Epub 2015 Jan 13.

Division of Pediatric Hematology/Oncology/Stem Cell Transplantation, Department of Pediatrics, Columbia University Medical Center, New York, New York.

Background: Vitamin D is increasingly recognized for its roles in non-skeletal disorders. Patients with sickle cell disease (SCD) have a high prevalence of vitamin D deficiency but data are limited with respect to possible associations between low vitamin D and acute vaso-occlusive complications. We examined whether vitamin D deficiency is associated with acute pain and acute chest syndrome (ACS) in children with SCD. Read More

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http://dx.doi.org/10.1002/pbc.25399DOI Listing
April 2015
7 Reads

Liver iron overload assessment by MRI R2* relaxometry in highly transfused pediatric patients: an agreement and reproducibility study.

Diagn Interv Imaging 2015 Mar 19;96(3):259-64. Epub 2014 Dec 19.

Service d'imagerie médicale, centre de référence de la drépanocytose, centre hospitalier intercommunal de Créteil, 40, avenue de Verdun, 94010 Créteil cedex, France.

Aims: Perform an agreement and reproducibility study of the estimation of iron overload in highly transfused pediatric patients comparing R2* relaxometry (R2*=1000/T2*) to the reference technique liver/muscle signal intensity ratio (SIR).

Patients And Methods: Ninety-two MRI were performed in 68 children who were mainly transfused for sickle cell disease, mean age 9.9 years old. Read More

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http://dx.doi.org/10.1016/j.diii.2014.11.021DOI Listing
March 2015
2 Reads

Musculoskeletal disorders in sickle cell anaemia--unusual associations.

J Assoc Physicians India 2014 Jan;62(1):52-3

Sickle cell anaemia coexisting with gout is a rare clinical association, as is gout and eosinophilia. This report records the second case of chronic tophaceous deposits in Sickle cell anaemia. The patient also had eosinophilia in association with gout. Read More

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January 2014
5 Reads

Arginine butyrate per os protects mdx mice against cardiomyopathy, kyphosis and changes in axonal excitability.

Neurobiol Dis 2014 Nov 26;71:325-33. Epub 2014 Aug 26.

CNRS, Institut de Neurobiologie Alfred Fessard, FRC2118, Neurobiologie & Développement, UPR 3294, Gif sur Yvette, F-91198, France. Electronic address:

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease caused by lack of dystrophin, a sub-sarcolemmal protein, which leads to dramatic muscle deterioration. We studied in mdx mice, the effects of oral administration of arginine butyrate (AB), a compound currently used for the treatment of sickle cell anemia in children, on cardiomyopathy, vertebral column deformation and electromyographic abnormalities. Monthly follow-up by echocardiography from the 8th month to the 14th month showed that AB treatment protected the mdx mice against drastic reduction (20-23%) of ejection fraction and fractional shortening, and also against the ≈20% ventricular dilatation and 25% cardiac hypertrophy observed in saline-treated mdx mice. Read More

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http://dx.doi.org/10.1016/j.nbd.2014.08.023DOI Listing
November 2014
12 Reads

Sickle cell disease with osteogenesis imperfecta.

J Assoc Physicians India 2013 Jun;61(6):415-7

A 16 yr old female presented with generalized weakness and easy fatigability since 2 months. Her medical history included that she had sickle cell disease (ss pattern) on regular treatment. She denied smoking and consumption of alcohol. Read More

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June 2013
6 Reads

Craniofacial features of patients with sickle cell anemia and sickle cell trait.

Angle Orthod 2014 Sep 4;84(5):825-9. Epub 2014 Mar 4.

a  Professor Orthodontics, Department of Health I, Southwest Bahia State University, Jequié, Bahia, Brazil.

Objective: To identify the craniofacial characteristics of patients with sickle cell trait (SCT) and sickle cell anemia (SCA) and to compare these measurements with those of nonaffected subjects.

Materials And Methods: Clinically normal patients and those with SCT and SCA were evaluated in this study. The patients were divided into three groups: normal (control), SCA, and SCT (n  =  with 15 in each group). Read More

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http://dx.doi.org/10.2319/101513-764.1DOI Listing
September 2014
5 Reads

Association between rs267196 and rs267201 of BMP6 gene and osteonecrosis among sickle cell aneamia patients.

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2015 Mar 13;159(1):145-9. Epub 2013 Nov 13.

Laboratory of Molecular and Cellular Hematology, Tunis El Manar University, Pasteur Institute of Tunis, Tunis, Tunisia.

Aims: The skeletal manifestations of sickle cell disease are the result of changes in bone and bone marrow caused by chronic tissue hypoxia that is exacerbated by episodic occlusion of the microcirculation by the abnormal sickle cells. Furthermore, the occurrence of osteonecrosis is under the control of some modifier gene. BMP6 (Bone morphogenetic protein) has been reported as associated with osteonecrosis in sickle cell anemia (SCA). Read More

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http://dx.doi.org/10.5507/bp.2013.080DOI Listing
March 2015
12 Reads

Beyond diet reconstruction: stable isotope applications to human physiology, health, and nutrition.

Am J Hum Biol 2013 Jul-Aug;25(4):445-56

Department of Anthropology, University of Georgia, Athens, GA 30602, USA.

Analysis of stable carbon and nitrogen isotopes from soft or mineralized tissues is a direct and widely-used technique for modeling diets. In addition to its continued role in paleodiet analysis, stable isotope analysis is now contributing to studies of physiology, disease, and nutrition in archaeological and living human populations. In humans and other animals, dietary uptake and distribution of carbon and nitrogen among mineralized and soft tissue is carried out with varying efficiency due to factors of internal biology. Read More

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http://dx.doi.org/10.1002/ajhb.22398DOI Listing
November 2013
3 Reads

Factors influencing outcome and incidence of late complications in children who underwent allogeneic hematopoietic stem cell transplantation for hemoglobinopathy.

Pediatr Hematol Oncol 2012 Nov 28;29(8):694-703. Epub 2012 Sep 28.

Department of Pediatric Hematology Oncology, Meyer Children's Hospital, Rambam Health Care Campus, Haifa, Israel.

Background: Hematopoietic stem cell transplantation (HSCT) remains the only potentially curative treatment for severe hemoglobinopathy (HGP). Late complications (LCs) are all events occurring beyond two years post-HSCT. We retrospectively analyzed prevalence, factors influencing occurrence, and prognosis of LCs post-HSCT for HGP. Read More

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http://dx.doi.org/10.3109/08880018.2012.725198DOI Listing
November 2012
8 Reads

Adrenergic nerves govern circadian leukocyte recruitment to tissues.

Immunity 2012 Aug 2;37(2):290-301. Epub 2012 Aug 2.

Ruth L. and David S. Gottesman Institute for Stem Cell Biology and Regenerative Medicine, Albert Einstein College of Medicine, New York, NY 10461, USA.

The multistep sequence leading to leukocyte migration is thought to be locally regulated at the inflammatory site. Here, we show that broad systemic programs involving long-range signals from the sympathetic nervous system (SNS) delivered by adrenergic nerves regulate rhythmic recruitment of leukocytes in tissues. Constitutive leukocyte adhesion and migration in murine bone marrow (BM) and skeletal-muscle microvasculature fluctuated with circadian peak values at night. Read More

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http://dx.doi.org/10.1016/j.immuni.2012.05.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3428436PMC
August 2012
12 Reads

Tuberculous spondylodiscitis in a patient with a sickle-cell disease: CT findings.

Pol J Radiol 2012 Jan;77(1):72-6

1 Department of Clinical Radiology of the Medical University of Warsaw, Warsaw, Poland.

Background: Although sickle-cell anemia (SCA) is common in black Americans, Sub-Saharan Africa and in the Mediterranean area, the disease is rare in the temperate climate zone. The manifestations of the disease are related mainly to the production of abnormal hemoglobin that leads to organ ischemia and increased susceptibility to infection caused by functional asplenia.

Case Report: The authors present CT findings in a 39-year-old black woman diagnosed due to abdominal pain, lymphadenopathy and fever. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3389959PMC
January 2012
14 Reads

Effects of regular physical activity on skeletal muscle structural, energetic, and microvascular properties in carriers of sickle cell trait.

J Appl Physiol (1985) 2012 Aug 14;113(4):549-56. Epub 2012 Jun 14.

Université de Savoie, Laboratoire de Physiologie de l'Exercice EA4338, Chambéry, France.

To assess the effects of regular physical activity on muscle functional characteristics of carriers of sickle cell trait (SCT), 39 untrained (U) and trained (T) hemoglobin (Hb)AA (CON) and SCT subjects (U-CON, n = 12; U-SCT, n = 8; T-CON, n = 10; and T-SCT, n = 9) performed a graded exercise and a time to exhaustion (T(ex)) test, and were subjected to a muscle biopsy. Maximal power, total work performed during T(ex), citrate synthase and cytochrome c oxidase (COX) activities, respiratory chain complexes I and IV content, and capillary density (CD), diameter (COD), and surface area (CSA) were upregulated by the same proportion in T-CON and T-SCT compared with their untrained counterparts. These proportionally similar differences imply that the observed discrepancies between U-SCT and U-CON remained in the trained subjects. Read More

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http://dx.doi.org/10.1152/japplphysiol.01573.2011DOI Listing
August 2012
25 Reads

Framing the research agenda for sickle cell trait: building on the current understanding of clinical events and their potential implications.

Am J Hematol 2012 Mar 3;87(3):340-6. Epub 2012 Feb 3.

Blood Diseases Branch, Division of Blood Diseases and Resources, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892-7950, USA.

Sickle Cell Trait (HbAS), the heterozygous state for the sickle hemoglobin beta globin gene is carried by as many as 100 million individuals including up to 25% of the population in some regions of the world (World Health Organization, Provisional agenda item 4.8, EB117/34 (22 December 2005) or World Health Organization, Provisional agenda item 11.4 (24 April 2006)). Read More

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http://dx.doi.org/10.1002/ajh.22271DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3513289PMC
March 2012
8 Reads

Exercise limitation, exercise testing and exercise recommendations in sickle cell anemia.

Clin Hemorheol Microcirc 2011 ;49(1-4):151-63

Laboratory ACTES, Department of Physiology, University of the French West Indies, Pointe a Pitre, Guadeloupe, French West Indies.

Sickle cell anemia (SCA or SS homozygous sickle cell disease) is an inherited blood disorder caused by single nucleotide substitution in the β-globin gene that renders their hemoglobin (HbS) much less soluble than normal hemoglobin (HbA) when deoxygenated. The polymerization of HbS upon deoxygenation is the basic pathophysiologic event leading to RBC sickling, hemolysis, vasoocclusion and ultimately to chronic organ damage. The metabolic changes imposed by exercise may initiate sickling and vaso-occlusive episodes. Read More

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http://www.researchgate.net/profile/Olivier_Hue2/publication
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http://dx.doi.org/10.3233/CH-2011-1465DOI Listing
May 2012
3 Reads

Skeletal scintigraphy manifestations of hematologic disorders.

Indian J Nucl Med 2012 Jan;27(1):59-62

Nuclear Medicine Services, Kothrud, Pune, Maharashtra, India.

Skeletal manifestations are common in hematologic disorders. Benign entities such as Sickle cell disease develop microvascular embolization causing skeletal crisis. Leukemia, acute myeloblastic or lymphoblastic may develop bone marrow infarcts. Read More

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http://dx.doi.org/10.4103/0972-3919.108880DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3628269PMC
January 2012
2 Reads

Orthodontic treatment in a patient with sickle cell anemia.

Am J Orthod Dentofacial Orthop 2011 Nov;140(5):713-9

Southwest Bahia University UESB, Jequié, Bahia, Brazil.

Sickle cell anemia is a common hereditary hematologic disease. It affects many systems and tissues in the body, including the mouth. Delayed tooth eruption, an uncommon degree of periodontitis, alterations in the cells of the tongue surface, hypomaturation and hypomineralization in enamel and dentin, pulp calcifications, hypercementosis, and bone alterations resulting in maxillary protrusion and formation of a thick trabecular pattern are some oral manifestations of the disease. Read More

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http://dx.doi.org/10.1016/j.ajodo.2010.02.039DOI Listing
November 2011
2 Reads

Sudden exertional death in sickle cell trait.

Br J Sports Med 2012 Apr 30;46(5):312-4. Epub 2011 Sep 30.

UCL Institute for Sport, Exercise and Health, Rosenheim Building, 25 Grafton Way, London WC1E 6DB, UK.

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http://dx.doi.org/10.1136/bjsports-2011-090521DOI Listing
April 2012
2 Reads

Brief communication: oxygen isotopes as a biomarker for sickle-cell disease? Results from transgenic mice expressing human hemoglobin S genes.

Am J Phys Anthropol 2011 Jul 3;145(3):495-8. Epub 2011 May 3.

Department of Anthropology, The Ohio State University, Columbus, 43210-1106, USA.

The origins of sickle-cell disease (SCD) are well understood, as are its evolutionary pressures on humans and pathological presentation. However, because it has not been possible to identify SCD in archaeological contexts, its biocultural effects on past populations are unknown. Previous research investigating oxygen isotope fractionation during respiration among anemics suggests that oxygen isotopes in bone apatite may provide a biological marker for SCD in skeletal remains. Read More

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http://dx.doi.org/10.1002/ajpa.21513DOI Listing
July 2011
3 Reads

Osteocalcin and specific markers of bone resorption in sickle cell disease.

Acta Physiol Hung 2010 Sep;97(3):290-6

Lagos State University, Department of Chemical Pathology, College of Medicine, Lagos, Nigeria.

Sickle cell disease commonly causes avascular necrosis of bone. Plain radiographs are not useful if obtained early in the disease. Radionuclide scans do not appear to increase the sensitivity of the diagnosis. Read More

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http://dx.doi.org/10.1556/APhysiol.97.2010.3.5DOI Listing
September 2010
2 Reads
0.750 Impact Factor

Granulomatous salmonella osteomyelitis associated with anti-tumor necrosis factor therapy in a non-sickle cell patient: a case report.

Skeletal Radiol 2010 Aug 21;39(8):821-5. Epub 2010 Feb 21.

Department of Radiology, State University of New York at Stony Brook, Stony Brook, NY 11794-8460, USA.

Salmonella osteomyelitis is seen most commonly in patients with sickle cell disease and in those with compromised immune systems. We report on the clinical, histological and imaging findings of salmonella osteomyelitis with intraosseous abscess formation occurring in a non-sickle cell patient receiving anti-tumor necrosis factor (TNF) alpha therapy. Read More

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http://link.springer.com/10.1007/s00256-010-0894-4
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http://dx.doi.org/10.1007/s00256-010-0894-4DOI Listing
August 2010
5 Reads

Salmonella pyomyositis complicating sickle cell anemia: a case report.

J Med Case Rep 2010 Jun 30;4:198. Epub 2010 Jun 30.

Department of Microbiology, Queen's Medical Centre, Derby Road, Nottingham, NG7 2UH, UK.

Introduction: Pyomyositis is a bacterial infection of skeletal muscle and a rare complication of sickle cell anemia. It may present a difficult problem in diagnosis, leading to delay in appropriate treatment and development of complications including abscess formation and osteomyelitis.

Case Presentation: We report the case of a 44-year-old Afro-Caribbean woman with homozygous sickle cell disease who presented with chest crisis and later developed pyomyositis of her hip and pelvic muscles. Read More

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http://dx.doi.org/10.1186/1752-1947-4-198DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2904793PMC
June 2010
3 Reads

Skeletal muscle structural and energetic characteristics in subjects with sickle cell trait, alpha-thalassemia, or dual hemoglobinopathy.

J Appl Physiol (1985) 2010 Sep 24;109(3):728-34. Epub 2010 Jun 24.

Laboratoire de Physiologie de l'Exercice EA4338, Université de Savoie, Chambéry, France.

Previous studies have shown that subjects with sickle cell trait (SCT), alpha-thalassemia (alpha-t), and the dual hemoglobinopathy (SCT/alpha-t) manifest subtle, albeit significant, differences during exercise. To better understand such differences, we assessed skeletal muscle histomorphological and energetic characteristics in 10 control HbAA subjects (C), 5 subjects with alpha-t (alpha-t), 6 SCT carriers (SCT) and 9 SCT carriers with alpha-t (SCT/alpha-t). Subjects underwent a muscle biopsy and also performed an incremental maximal exercise and a time to exhaustion test. Read More

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http://www.physiology.org/doi/10.1152/japplphysiol.00349.201
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http://dx.doi.org/10.1152/japplphysiol.00349.2010DOI Listing
September 2010
12 Reads

Spectrum of musculo-skeletal disorders in sickle cell disease in Lagos, Nigeria.

J Orthop Surg Res 2010 Jan 18;5. Epub 2010 Jan 18.

Department of Surgery, College of Medicine, University of Lagos (CMUL)/Lagos University Teaching Hospital (LUTH), PMB 12003, Lagos, Nigeria.

Background: Sickle cell anemia (SCA) is a common genetic disease in Nigeria. Past studies from West Africa focused on isolated aspects of its medical and surgical presentations. To the best of our knowledge, the musculo-skeletal presentations amongst Nigerians with SCA have not been documented in a single all encompassing study. Read More

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http://dx.doi.org/10.1186/1749-799X-5-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2821293PMC
January 2010
2 Reads

Exertional rhabdomyolysis and acute kidney injury.

Phys Sportsmed 2009 Apr;37(1):71-9

Primary Care Sports Medicine Program, Michigan State University, Kalamazoo, MI 49008, USA.

Skeletal muscle breakdown occurs normally with exercise, followed by muscle repair and physiologic adaptation. Strenuous, unaccustomed, prolonged, and repetitive exercise, particularly when associated with other risk factors such as hot and humid climate or sickle cell trait can cause clinically significant exertional rhabdomyolysis (ER). Although most cases are asymptomatic and resolve without sequelae, ER is the most common cause of exercise-related myoglobinuric acute renal injury and acute renal failure in athletes. Read More

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http://dx.doi.org/10.3810/psm.2009.04.1685DOI Listing
April 2009
58 Reads

Multidetector CT scan in the evaluation of chest pain of nontraumatic musculoskeletal origin.

Radiol Clin North Am 2010 Jan;48(1):185-91

Division of Diagnostic Radiology, Section of Musculoskeletal Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO 63110, USA.

Acute nontraumatic chest pain is a common presenting symptom to the emergency department. Often, it is evaluated by thin-collimation multidetector computed tomography scan (MDCT) using pulmonary embolism, aortic dissection, or coronary artery protocols. The parameters used for these protocols are very similar to those used in protocols for dedicated imaging of the musculoskeletal system. Read More

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http://dx.doi.org/10.1016/j.rcl.2009.09.007DOI Listing
January 2010
4 Reads

Remodeling of skeletal muscle microvasculature in sickle cell trait and alpha-thalassemia.

Am J Physiol Heart Circ Physiol 2010 Feb 13;298(2):H375-84. Epub 2009 Nov 13.

Laboratoire de Physiologie de l'Exercice, Université de Savoie, Chambéry, France.

The influence of sickle cell trait and/or alpha-thalassemia on skeletal muscle microvascular network characteristics was assessed and compared with control subjects [hemoglobin (Hb) AA] in 30 Cameroonian residents [10 HbAA, 5 HbAA alpha-thalassemia (alpha-t), 6 HbAS, and 9 HbASalpha-t] matched for maximal work capacity and daily energy expenditure. Subjects performed an incremental exercise to exhaustion and underwent a muscle biopsy. Muscle fiber type and surface area were not different among groups. Read More

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http://dx.doi.org/10.1152/ajpheart.00812.2009DOI Listing
February 2010
12 Reads

In vivo pharmaco-proteomic analysis of hydroxyurea induced changes in the sickle red blood cell membrane proteome.

J Proteomics 2010 Jan 13;73(3):619-26. Epub 2009 Nov 13.

Department of Molecular and Cell Biology, University of Texas at Dallas, Richardson, Texas 75083-0688, USA.

Hydroxyurea (HU) is an effective drug for the treatment of sickle cell disease (SCD). The main clinical benefit of HU is thought to derive from its capacity to increase fetal hemoglobin (HbF) production. However, other effects leading to clinical benefit, such as improved blood rheology, have been suggested. Read More

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http://dx.doi.org/10.1016/j.jprot.2009.11.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2818491PMC
January 2010
3 Reads
3.890 Impact Factor

Enumeration of the colony-forming units-fibroblast from mouse and human bone marrow in normal and pathological conditions.

Stem Cell Res 2009 Jan 12;2(1):83-94. Epub 2008 Aug 12.

Department of Health and Human Services, Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892-4370, USA.

Bone marrow stromal cell populations, containing a subset of multipotential skeletal stem cells, are increasingly contemplated for use in tissue engineering and stem cell therapy, whereas their involvement in the pathogenetic mechanisms of skeletal disorders is far less recognized. We compared the concentrations of stromal clonogenic cells, colony forming units-fibroblast (CFU-Fs), in norm and pathology. Initially, culture conditions were optimized by demonstrating that fetal bovine serum heat inactivation could significantly repress colony formation. Read More

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http://dx.doi.org/10.1016/j.scr.2008.07.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2753860PMC
January 2009
3 Reads